Cyanosis or Congestive Heart Failure in Children: Murmurs of Shunts, Stenosis, and Insufficiency A....
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Transcript of Cyanosis or Congestive Heart Failure in Children: Murmurs of Shunts, Stenosis, and Insufficiency A....
Cyanosis or Congestive Heart Failure in Children:
Murmurs of Shunts, Stenosis, and Insufficiency
A. Dodge-Khatami, MD, PhDChief of Pediatric Cardiac SurgeryHead of Program for Congenital Heart DiseaseUniversity Heart Center – UHZUniversity of Hamburg-Eppendorf School of MedicineHamburg, Germany
Klinik für Kinderherzchirurgie
even rare congenital heart defects will be seen once in your careers (0.8% of all births); how should you react?
most important objective:
distinguish between a blue and pink patient with a murmur and understand why!
Klinik für Kinderherzchirurgie
Shunts: Location + Direction
• Intra or extra-cardiac? • Which heart chambers are
affected?• Qp/Qs = pulmonary / systemic
flow ratio
Qp = VO2 / pulm Vv O2 – PA O2
Qs = VO2 / Vv O2 – Ao O2
• In the absence of a shunt, Qp/Qs = 1
Klinik für Kinderherzchirurgie
Normal circulation
Klinik für Kinderherzchirurgie
Qp/Qs = 1Q = P/R
Shunts: Direction
• Left >>> right or Right >>> left?
Which is more probable? Why?
• Left > right : PDA, ASD, VSD, AVSD, AP window, Truncus, PAPVD,
TAPVD
• Right > left : right inflow or outflow obstruction + intra-cardiac
shunt: Tricuspid atresia (TA)/Tricuspid Stenosis (TS), Pulmonary
Atresia/Pulmonary stenosis, TOFallot
Klinik für Kinderherzchirurgie
Shunts: Direction
• Left >>> right : VSD
Klinik für Kinderherzchirurgie
Left >> right shuntQp/Qs > 2 - 3
Pressure + Volume Overload
Shunts: Physiology
Left >>> right:• LV volume overload• Increased pulmonary flow, pulmonary infections• Pulmonary Hypertension (PHN), severity and degree
according to shunt size• Bacterial endocarditis
Right >>> left:• RV pressure overload + strain• Cyanosis• Polyglobulia
Klinik für Kinderherzchirurgie
Shunts: Treatment
Left >>> right:
volume restriction, diuretics, inotropes, permissive
hypercapnea ventilation (hypoventilation),
shunt closure
Right >>> left:
hydration, (transfusion), hyperventilation, increase
pulmonary blood flow +/- shunt closure
Klinik für Kinderherzchirurgie
Shunts: Operative Indications
L >> R:• Symptoms: tachycardia, tachypnea, hepatomegaly,
sweating during feeds, failure to thrive• Qp:Qs > 1.5• Aortic valve prolapse +/- insufficiency
R >> L:• cyanosis, RVH + strain
Klinik für Kinderherzchirurgie
5 most common congenital heart defects?
Klinik für Kinderherzchirurgie
5 most common congenital heart defects?
Ventricular Septal Defect (VSD) 30%
Patent Ductus Arteriosus (PDA) 10%
Coarctation (coA) 5-8%
Atrial Septal Defect (ASD) ~ 8%
Tetralogy of Fallot (TOF) 5-10%
Klinik für Kinderherzchirurgie
• case: blue child (10 years old) with a murmur (where?)auscultation: holosystolic murmur at precordiumsaturations: ? Cyanosis: central or peripheral?
Central:
intracardiac shunt + obstruction to pulmonary blood flow
Peripheral:
Chronic Pneumonia, Chronic Interstitial Lung Disease, Pulmonary
Neoplasia, Circulatory Collapse (+Peripheral Vasoconstriction)
next step ?
Klinik für Kinderherzchirurgie
„Hippocratic fingers“- Clubbing
x-ray:
differential diagnosis?
Klinik für Kinderherzchirurgie
x-ray: • prominent central pulmonary markings• black peripheral lung fields
next step ?
Klinik für Kinderherzchirurgie
echocardiography:
Cardiomegaly, biventricular dilatation + hypertrophy
Diagnosis ?
Klinik für Kinderherzchirurgie
echocardiography:
Cardiomegaly, biventricular dilatation + hypertrophy
VSD: why is the child blue?
Klinik für Kinderherzchirurgie
Right >> Left shunting = Cyanosis
>
increased cellularity (muscular and interstitial)>> fixed pulmonary vascular resistance = Eisenmenger syndrome
Klinik für Kinderherzchirurgie
Patent Ductus Arteriosus
(PDA)
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Patent Ductus Arteriosus
(PDA)
Klinik für Kinderherzchirurgie
• continuous „machinery“ murmur
• LV hypertrophy + LA dilatation Increased pulmonary vascular markings, interstitial pulmonary edema
• failure to thrive
• recurrent upper respiratory infections
• fatigue with exertion
• tachypnea, tachycardia, heart failure
•
Patent Ductus Arteriosus
(PDA)
Klinik für Kinderherzchirurgie
R. Gross, Boston, 1938
Portsmann, 1967
Coarctation
(coA)
Klinik für Kinderherzchirurgie
Coarctation
(coA)
Klinik für Kinderherzchirurgie
• bi-modal presentation:
newborns in cardiovascular shock: ductal-dependent (PGE1)
vs.
„asymptomatic“ hypertensive children: headaches, epistaxis
Coarctation
(coA)
Klinik für Kinderherzchirurgie
• mid-systolic murmur in the back, systolic or continuous murmurs on the lateral chest walls (collaterals), diminished femoral pulses
• Left Ventricular hypertrophy, myocardial infarction
• circle of Willis aneurysms, aortic aneurysms, aortic dissection, aortic rupture
• average age at death ~ 35 years if untreated : congestive heart failure (1/4), bacterial endocarditis (1/4), spontaneous rupture of the aorta (20%), intracranial hemorrhage (13%)
Coarctation
(coA)
Klinik für Kinderherzchirurgie
C. Crafoord, Stockholm, 1944
End-to-end anastomosis
Coarctation
(coA)
Klinik für Kinderherzchirurgie
Voßschulte, 1957Patch plasty
Gross, 1951Interposition graft
Waldhausen, 1966Subclavian flap
Coarctation
(coA) : results
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Mortality: 4-14%, age-dependent
Complications:
• hypertension, chylothorax, recurrent nerve paresis (stridor)
• recurrent coA ~ 10-15% if surgery in the newborn period, >> balloon dilatation
• paraplegia
• aneurysm
Atrial Septal Defect
(ASD)
Klinik für Kinderherzchirurgie
• systolic murmur, fixed split second heart sound (prolonged flow time on the right – delayed closure of the pulmonary valve)
• Dilated right atrium + ventricle
• Pulmonary hypertension recurrent upper respiratory infections
• atrial arrhythmia (flutter, fibrillation)
• congestive heart failure
• no risk of bacterial endocarditis
Atrial Septal Defect
(ASD)
Klinik für Kinderherzchirurgie
F.J. Lewis, Minneapolis, 1952, inflow occlusion
King, 1976, device closure
Atrial Septal Defect
(ASD)
Klinik für Kinderherzchirurgie
J. Gibbon Jr., Rochester, father of cardio-pulmonary bypass, 1934-53
Atrial Septal Defect
(ASD)
Klinik für Kinderherzchirurgie
J. Gibbon Jr., Rochester, 1953
Atrial Septal Defect
(ASD) : results
Klinik für Kinderherzchirurgie
• Gibbon (1953): first success, followed by 5 deaths, abandonned surgery and requested a 1 year moratorium on his bypass machine…
• current: mortality ~ 0%
Ventricular Septal Defect
(VSD)
most frequent CHD ~ 30%
Klinik für Kinderherzchirurgie
Ventricular Septal Defect
(VSD)
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• Holosystolic murmur, increased pulmonary vascularity on x-ray,
• Cardiomegaly, biventricular dilatation + hypertrophy.
• Dyspnea, sweating during feeding, failure to thrive.
• Recurrent upper respiratory tract infections.
Ventricular Septal Defect
(VSD)
Klinik für Kinderherzchirurgie
Untreated:
• 25-40% spontaneous closure > 2-3 years
• endocarditis (0.3% per year)
• pulmonary hypertension > pulmonary arteriolar wall thickening
• increased PVR, reversal of shunt
= Eisenmenger syndrome
• cyanosis (by 1-2 years of age)
• death
Ventricular Septal Defect
(VSD)
Klinik für Kinderherzchirurgie
Ventricular Septal Defect
(VSD >>> VSD)
Klinik für Kinderherzchirurgie
• increased cellularity (muscular and interstitial)
• increased reactivity• fixed contraction• vascular wall sclerosis
>> fixed pulmonary vascular resistance = Eisenmenger syndrome
Ventricular Septal Defect
(VSD)
Klinik für Kinderherzchirurgie
C.W. Lillehei, Minneapolis1954: VSD„King of Hearts: the True Story of the Maverick Who Pioneered Open Heart Surgery “, G.W. Miller
Cross-circulation: father as oxygenator, but potentially 200% mortality…
Ventricular Septal Defect
(VSD)
Klinik für Kinderherzchirurgie
C.W. Lillehei, Minneapolis1954: VSD28/47 patients survived:~ 40% mortality
Ventricular Septal Defect
(VSD)
Klinik für Kinderherzchirurgie
Ventricular Septal Defect
(VSD): Results
mortality ~ 1-2%
heart block > pacemaker 1-2%
Klinik für Kinderherzchirurgie
long-term prognosisexcellent!
Ventricular Septal Defect
(VSD): palliation
Klinik für Kinderherzchirurgie
PA banding• multiple VSDs
• small baby, failure to thrive
Muller / Damman, 1952
Tetralogy of Fallot (TOF)most frequent cyanotic CHD ~ 10%
1. Overriding Aorta
2. Ventricular Septal Defect
3. Right ventricular hypertrophy
4. Right Ventricular Outflow Tract
Obstruction (RVOTO)
Klinik für Kinderherzchirurgie
Klinik für Kinderherzchirurgie
Tetralogy of Fallot
• systolic murmur
• right aortic arch (25%), „boot shape“ heart
• right ventricular hypertrophy
• cyanosis, tet „spells“: dynamic RVOT contraction
• clubbing (after 6 months), dyspnea, exercise intolerance
• brain abscess
• polycythemia > pulmonary + cerebral thrombosis
Tetralogy of Fallot (TOF)
Palliation
Klinik für Kinderherzchirurgie
H. Taussig A. Blalock
Baltimore, 1944, classic Blalock-Taussig Shunt = „blue baby operation“
Modified BT shunt, 1976
Tetralogy of Fallot (TOF)
Right Ventricular Outflow Tract Obstruction (RVOTO):
- Suprapulmonary (Pulmonary Arteries)
- Pulmonary Valve
- Subpulmonary (Right Ventricle)
Central Importance of the Pulmonary Valve
distally: Pulmonary Artery growth
proximally: protect the Right Ventricle
Klinik für Kinderherzchirurgie
Tetralogy of Fallot
(TOF): complete repair
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C.W. Lillehei, Minneapolis1955: Fallot correction
Tetralogy of Fallot
(TOF)
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Tetralogy of Fallot
(TOF): results
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Mortality 3-5 %
Heart Block < 3%, seldom requires a pacemaker
Post-operative arrhythmia frequent
Reoperations required for:
• residual VSD (seldom)• residual pulmonary valve insufficiency• residual right outflow obstruction
Tetralogy of Fallot
(TOF): reoperations
Klinik für Kinderherzchirurgie
• residual pulmonary valve INSUFFICIENCY
right ventricular volume overload + dilatation + failurearrhythmiabetter growth of pulmonary arteries?late REOPERATION
• residual right outflow STENOSIS
right ventricular pressure overloadpulmonary artery stenosis/hypoplasialate REOPERATION